A case of scleroderma renal crisis with acute interstitial pneumonia, microangiopathic hemolytic anemia and refractory thrombocytopenia

A 46-year-old woman who noticed tightness of the skin in September, 1993, was admitted to a local hospital due to hypertension, congestive heart failure and renal dysfunction on the 2nd of November. After admission, renal function deteriorated progressively. A diagnosis of scleroderma renal crisis (SRC) was suspected from her skin biopsy and clinical course. She was referred to our hospital for further evaluation and maintenance of hemodialysis. Her blood pressure was kept normal by anti-hypertensive drugs including cilazapril. Acute interstitial pneumonia, microangiopathic hemolytic anemia and thrombocytopenia appeared during her clinical course. Corticosteroid therapy was effective for acute interstitial pneumonia, but in-effective for thrombocytopenia. Plasma exchange was not effective for thrombocytopenia, which was successfully treated with intravenous γ-globulin therapy. She died of cytomegaloviral encephalitis, which might have resulted from immunodeficiency caused by prolonged corticosteroid therapy and uremia. Complications other than SRC might have appeared during the clinical course based on the immune disorder of progressive systemic sclerosis itself. In order to improve the prognosis of patients with SRC such complications should be detected promptly and treated correctly.